摘要:

Six cases of constitutional aplastic anemia (Fanconi's anemia), including four boys and two girls who were between 6 and 10 years of age, are presented. Congenital abnormalities were observed in all the patients, especially skin pigmentations, renal anomalies, and growth retardation. The initial symptoms were insidious, mainly related to the anemia. All patients were anemic and thrombocytopenic, but only three were leukopenic. Five cases showed increased levels of fetal hemoglobin, which was marked in two. Bone marrow obtained by aspiration and/or biopsy was hypocellular in all cases. Chromosomal studies showed breaks, ring- or cross-chromosomes, chromosome exchanges, and endoreduplications. Five cases were treated with androgens and a good response was obtained in all of them. Four patients died, and the remaining two are alive and well. The clinical and biological features of these patients are in accordance with those of previous reports. The importance of the an-drogen therapy is stressed; androgen dependence was observed in all cases.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

The properties of the recombinant DNA-derived human leukocyte interferon, HuIFNα2, were studied in patients with advanced leukemia or lymphoma. In vitro, HuIFNα2induced an increased activity of 2–5A synthe-tase in leukemic and in control cells indicating cellular responsiveness to IFN. HuIFNα2also produced a dose-responsive decline in marrow leukemia blast progenitor colony growth, and in normal hematopoietic colony formation in vitro, confirming its antiproliferative effect. A course of intravenous therapy given to a lymphoma patient produced a modest decline in peripheral white blood cell (WBC) and neutrophil counts; higher, more frequent doses in a second patient induced a profound drop in WBC's, neutrophils, and platelets. When the leukemia patients were given an intravenous course of HuIFNα2as a sole agent, blast cytoreduction was seen in peripheral blood in three patients, and in marrow of one patient with acute myeloblastic leukemia (AML). Elevated 2–5A synthetase levels could be detected after therapy. No modulation of leukemic cell markers was seen after in vitro or in vivo treatment with HuIFNα2, implying that the cytoreduction was not linked to blast cell differentiation. These studies suggest that this subtype of recombinant DNA-derived IFN has antileukemic properties, and indicates the possibilities for IFN as an adjunctive form of therapy in leukemia.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Thirty patients with Burkitt's lymphoma sporadic type are presented. All of these cases fulfilled the histologie criteria of Burkitt's tumor. The age ranged from 2 to 12 years, with a median of 4 years. Fever and chills were the most common symptoms. The abdomen was the site of initial presentation in more than half of these cases. Close to one-third of the patients had involvement of the central nervous system. Fifty percent of the patients presented with lactic dehydrogenase elevation. Uric acid was elevated in close to one-fourth of these patients. The sono-gram was the most useful technique in those instances of abdominal presentation. All patients were staged according to Ziegler criteria after an initial surgical diagnostic procedure was performed. None of the patients received radiotherapy to the primary tumor. All were submitted to a chemotherapy program for 12 months. Only 26 fitted the criteria of being évaluable. The duration of remission was from 1 to 144 months, with a median of 18.2 months. The actuarial survival was 84% at 3 years. Most of the responders corresponded to Stages A, B, and AR, while Stages C and D had a high mortality rate with elevated lactic dehydrogenase and uric acid levels.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

In some infants with Down's syndrome, the circulating mononuclear population, when viewed with conventional and electron microscopy, contains many cells that closely resemble leukemic blast cells. In contrast with true leukemia, however, most of these infants with the “leukemia like reaction in Down's syndrome” (LLR-DS) enter spontaneous remissions. We therefore investigated the natural resistance of such infants to hematological malignancy in vitro by means of natural killer cell assays. Mean natural killer cell determinations in four infants with LLR-DS were 17.5 ± 9.2% and 37.6 ± 18.5% against K-562 and Molt-4 target cells, respectively, at diagnosis. Later, during remission, these values were 34.3 ± 14.3% against K-562 and 32.2 ± 15.6% against Molt-4. The mean percentage lysis of Molt-4 both at diagnosis and during remission was greater (p < 0.05) in LLR-DS than in children with acute lymphocytic leukemia and acute myelogenous leukemia at diagnosis. Natural killer cell activity levels in these LLR-DS patients were similar to levels obtained in other infants with Down's syndrome who were hematologically normal, as well as levels obtained in normal control specimens. Two of these LLR-DS patients progressively developed acute myelogenous leukemia with ultrastructural abnormalities several months later; one of these also developed another karyotype abnormality. Both remain in long-term remission exceeding 48 months.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

This report describes a follow-up study in which the prevalence of emotional and behavioral problems in a group of 42 children and adolescents treated for leukemia is compared with the prevalence of problems in a matched control group selected from the general population. The prevalence of problems among the siblings of the two groups, and the adjustment of the two groups of families, are also examined. The Achenbach Child Behavior Checklist, for completion by parents, and the Achenbach Child Behavior Checklist and Rutter B2 Behavioural Scale, for completion by teachers, are used to identify both social competence and behavioral problems. In addition, the Family Concept Inventory is used to evaluate the adjustment of families. The leukemic children and adolescents were found to have significantly more problems and less social competence, particularly in school-related activities, than either the control group or their own siblings. There was no difference in the prevalence of problems between the two groups of siblings, nor between the two groups of families. It is suggested that careful prospective studies are needed to identify the cause of the problems experienced by the leukemic children and adolescents so that programs may be developed to prevent their emergence.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

The subject of analysis is a group of 111 children with acute lymphoid leukemia (ALL) whose remission has lasted at least 4 years since stopping treatment. Patients were observed from 4 to 18 years after ALL therapy. No symptoms of disease were observed in 110 children; one child had leukemic infiltration of the testes during the fifth year after stopping treatment. In this group of children no changes in physical development have been recorded, but a growth deficiency is sometimes noted. No symptoms of intrinsic organ lesions have been ascertained in most of the patients. All of the patients, except one with schizophrenia, lead normal lives and either attend school or go to work. Seven patients have healthy children, who were born 6–24 years after the beginning of the disease and 3–16 years after cessation of therapy.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Between 1974 and 1982, 43 children less than 2 years of age were treated in the hematology department of Hospital Saint-Louis for acute lymphoblastic leukemia (ALL). Of the patients who presented before 18 months of age, 80% had a WBC > 100,000 μl and/or a great tumor bulk. As a result of our experience, treatment regimens have been changed here from conventional chemotherapy to a very intensive program with a heavy induction (vincristine, daunorubicin, cyclophosphamide, prednisone, and L-asparaginase) and monthly reinductions with the same drugs plus ArA-C, without maintenance. Prophylaxis included CNS irradiation (16–24 Gy) after 12 months of age, plus intrathecal methotrexate. Complete remission (CR) occurred in 78% before 18 months and in 100% between 18 and 24 months of age at diagnosis. In this report the probability of a prolonged CR (33% at 2 years) was the same before and after 12 months of age. However, younger patients were more intensively treated. The prognosis for children less than 1 year of age who received very intensive chemotherapy has greatly improved, with a significantly higher probability of long CR (p < 0.02). Presently, 10 of 43 children are in CR 27 months to 8 years after diagnosis. Of 18 patients aged less than 1 year at diagnosis, four are in CR. No relapse occurred after 23 months. None of these patients presented with important sequellae, with the exception of one child who suffered from severe bacterial meningitis. An aggressive chemotherapy program is indicated in patients less than 2 years of age. The feasibility of this mode of treatment in young patients is possible only with the help of specific supportive care.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

At present, the majority of children with acute lymphoblastic leukemia can expect a favorable outcome. However, leukemia in infants (0–1 year) still runs a dismal course. The first year of life also has a unique pattern of hematopoietic activity. We investigated possible association between these two findings. We examined the characteristics of 51 infants with leukemia, diagnosed from 1975 to 1984; 48/51 had evaluable data. Infants had a high frequency (39.6%) of hyperleukocytosis (> 100 × 109/ liter), early central nervous system involvement (27.1%), and cutaneous infiltration (18.8%). The respective numbers in patients ≥1 year were 13.9, 4.8, and 1.0%. Acute nonlymphocytic leukemia was diagnosed in 18/48 infants (37.5%) compared to 128/892 (14.3%) in older patients. Infants had only acute leukemia of French-American-British (FAB) class M5 (10/19) or acute leukemia with unclassifiable but monocytoid blasts (8/19). This study confirms the presence of poor prognostic features in infant leukemia. The almost exclusive occurrence of monoblastic or monocytoid acute leukemia parallels the high proliferation rate of monocytes in this age group and the leukemic cells may thus well reflect frozen stages of monocytic differentiation.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Both nephroblastomatosis and deletions of the short arm of chromosome 11 (11p-) have been associated independently with Wilms' tumor. The finding of 11p- in a specimen of nodular renal blastema in the currently described patient represents a previously unknown association with this chromosomal lesion. The possibility that 11p- produced an abnormal renal substrate (nephroblastomatosis), upon which the action of a second postzygotic genetic alteration led to Wilms' tumor, is considered. It is suggested that, in the present case, tumorigenesis may have been the result of two postzygotic events, one of which may have been postnatal. Recent cytogenetic observations in both Wilms' tumor and retinoblastoma support such an hypothesis.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID